Biocomputing Group, Department of Pharmacy and Biotechnology, University of Bologna, Bologna, Italy.
CRO, Centro di Riferimento Oncologico, Aviano, Italy.
Sci Rep. 2022 Oct 26;12(1):17963. doi: 10.1038/s41598-022-22818-5.
According to databases such as OMIM, Humsavar, Clinvar and Monarch, 1494 human enzymes are presently associated to 2539 genetic diseases, 75% of which are rare (with an Orphanet code). The Mondo ontology initiative allows a standardization of the disease name into specific codes, making it possible a computational association between genes, variants, diseases, and their effects on biological processes. Here, we tackle the problem of which biological processes enzymes can affect when the protein variant is disease-associated. We adopt Reactome to describe human biological processes, and by mapping disease-associated enzymes in the Reactome pathways, we establish a Reactome-disease association. This allows a novel categorization of human monogenic and polygenic diseases based on Reactome pathways and reactions. Our analysis aims at dissecting the complexity of the human genetic disease universe, highlighting all the possible links within diseases and Reactome pathways. The novel mapping helps understanding the biochemical/molecular biology of the disease and allows a direct glimpse on the present knowledge of other molecules involved. This is useful for a complete overview of the disease molecular mechanism/s and for planning future investigations. Data are collected in DAR, a database that is free for search and available at https://dar.biocomp.unibo.it .
根据 OMIM、Humsavar、Clinvar 和 Monarch 等数据库,目前有 1494 个人类酶与 2539 种遗传疾病相关联,其中 75%是罕见疾病(有孤儿病代码)。Mondo 本体论倡议允许将疾病名称标准化为特定代码,从而可以在基因、变体、疾病及其对生物过程的影响之间进行计算关联。在这里,我们解决了当蛋白质变体与疾病相关时,酶可以影响哪些生物过程的问题。我们采用 Reactome 来描述人类的生物过程,并通过在 Reactome 途径中映射与疾病相关的酶,建立了 Reactome-疾病关联。这允许根据 Reactome 途径和反应对人类单基因和多基因疾病进行新的分类。我们的分析旨在剖析人类遗传疾病宇宙的复杂性,突出疾病和 Reactome 途径之间的所有可能联系。新的映射有助于理解疾病的生化/分子生物学,并允许直接了解其他涉及的分子的现有知识。这对于全面了解疾病的分子机制/和规划未来的研究很有用。数据收集在 DAR 数据库中,该数据库可免费搜索,网址为 https://dar.biocomp.unibo.it 。